Huntington`s Chorea - a Case Report and review of literature

A 45 years old gentleman presented to Medical Clinic of Naval Hospital (PNS Hafeez) Islamabad in January 2001 with choreform movements of the body and early dementia of six months duration. He had a strong family history of similar disease, which included his father and two brothers. On neurological examination he had choreoathetoid movements and features of early dementia. His chest was clinically clear and examination of heart and abdomen was normal. Eye examination did not reveal any evidence of Kayser-Fleischer rings (seen in Wilson`s disease). Laboratory investigations showed haemoglobin 13.8 Gm/dl, WBC 7.7 x10e9/L, serum urea 32 mg/dl, serum sodium 139 mmol/L serum potassium 3.8 mmol/L, ASO titre less than 200 IU/ml, serum bilirubin 6 umol/L, ALT 102 U/L, serum alkaline phosphatase 155 U/L, serum caeruloplasmin 42 mg/dl and serum copper 164 mmol/L (WNL). X-ray chest was normal and CT scan brain showed early generalized cerebral atrophy. He was managed with haloperidol (0.5mg) 12 hourly, Procyclidine (kemadrin, 5mg) 8 hourly and Propranolol (Inderal 10mg) 8 hourly. He was discharged from hospital with relative improvement after 2 weeks.